Cystic Fibrosis Primary Ciliary Dyskinesia
Conditions
Interventions
Longitudinal observational study during one year with collection of 5 exhaled breath and microbiology samples.
Sponsors
Dept. Respiratory Medicine
Academic Medical Centre, University of Amsterdam
Eligibility
Inclusion criteria
Inclusion criteria: 1. CF diagnosis is based on: clinical symptoms in combination with an abnormal sweat test (chloride > 60 mmol/l) and/or identification of mutations in both alleles of the CFTR gene; 2. PCD diagnosis is based on: A combination of clinical symptoms, abnormal movement of cilia on microscopic evaluation of respiratory epithelial biopsies and epithelial cell cultures, or identification of an ultra structural defect in the cilia by electron microscopy; 3. ≥ 6 years of age; 4. Stable respiratory disease for at least 6 weeks ( as determined by the treating physician); 5. Ability to perform lung function measurement.
Exclusion criteria
Exclusion criteria: 1. Mental retardation; 2. Diabetes Mellitus (CF complication); 3. Technical unsatisfactory performance of measurements; 4. On the waiting list for lung transplantation; 5. Participation in the PREVEC or VERTEX study (AMC).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Relative changes in electronic nose sensors; 2. Retention time; 3. Abundance and mass to charge ratio from GC-MS analysis. | — |
Secondary
| Measure | Time frame |
|---|---|
| (Change in) bacterial and viral diversity. | — |
Contacts
Public ContactT. Paff
Postbus 7057
Outcome results
None listed